Pasini Francesco, Karantzoulis Aristotelis, Fanella Gaia, Brovelli Francesco, Iacobucci Davide, Aprea Vittoria, Storti Benedetta, Santangelo Francesco, Canonico Francesco, Remida Paolo, Ferrarese Carlo, Brighina Laura
Department of Neurology, School of Medicine and Surgery, San Gerardo Hospital, ASST Monza, University of Milano-Bicocca, Monza, Italy.
Department of Neuroradiology, San Gerardo Hospital, ASST Monza, Monza, Italy.
Case Rep Neurol. 2023 Feb 7;15(1):24-30. doi: 10.1159/000528880. eCollection 2023 Jan-Dec.
Hyper-/hypoglycemic states are rare but well-established causes of hyperkinetic movements, including chorea and ballismus, usually associated with brain lesions in the basal ganglia. We report a case of hemichorea-hemiballismus (HCHB) syndrome that developed after a severe hypoglycemic episode in a 71-year-old man with poorly controlled type 2 diabetes mellitus. Uncommonly, brain MRI showed contralateral cortical-subcortical T2 and T2-FLAIR-hyperintense frontoparietal lesions, with cingulate gyrus involved, while the basal ganglia were unaffected. In patients with hypoglycemic encephalopathy associated with cortical lesions, the long-term prognosis is usually poor. Nevertheless, in our patient, the dyskinesias and the cerebral lesions progressively regressed by achieving good glycemic control. After four and 12 months, the patient's neurological examination was normal. To our knowledge, this is the first evidence of hypoglycemic etiology of cortical HCHB syndrome, supporting recent theories that cortical circuitries may independently contribute to the pathogenesis of chorea and ballismus. This is also the first report of cingulate gyrus involvement in hypoglycemic encephalopathy. Finally, this case may indicate that a subset of patients with cortical lesions due to hypoglycemia could present a good clinical outcome, likely depending on the size of the lesions and the duration and severity of the hypoglycemic episode.
高血糖/低血糖状态是导致运动增多性运动障碍的罕见但已明确的病因，包括舞蹈症和投掷症，通常与基底神经节的脑损伤有关。我们报告一例偏侧舞蹈症-偏侧投掷症（HCHB）综合征，该综合征发生在一名71岁、2型糖尿病控制不佳的男性严重低血糖发作之后。不同寻常的是，脑部MRI显示对侧皮质-皮质下T2加权像及T2液体衰减反转恢复序列（T2-FLAIR）上额顶叶高信号病变，累及扣带回，而基底神经节未受影响。在伴有皮质病变的低血糖性脑病患者中，长期预后通常较差。然而，在我们的患者中，通过实现良好的血糖控制，运动障碍和脑部病变逐渐消退。4个月和12个月后，患者的神经系统检查结果正常。据我们所知，这是皮质HCHB综合征低血糖病因的首个证据，支持了近期的理论，即皮质环路可能独立参与舞蹈症和投掷症的发病机制。这也是扣带回受累于低血糖性脑病的首例报告。最后，该病例可能表明，一部分因低血糖导致皮质病变的患者可能会有良好的临床结局，这可能取决于病变的大小以及低血糖发作的持续时间和严重程度。
